Subcorneal pustular dermatosis in the pediatric age

Abstract: Subcorneal pustular dermatosis is a rare pustular eruption which occurs mainly in middle-aged women and rarely during childhood. We report a case of a 15-year-old female with a 4-year history of pustular lesions on the proximal region of the upper limbs with subsequent impairment of the trunk. Physical examination revealed small pustules distributed on the trunk and proximal region of the limbs. Histopathology showed a subcorneal pustule and direct immunofluorescence for IgA, IgM, IgG and fibrinogen was negative, confirming the diagnosis of subcorneal pustular dermatosis. The patient was treated with dapsone with good clinical response after one month. Subcorneal pustular dermatosis is a rare condition and there are only isolated cases reported in the literature in pediatric patients. Thus, we discuss the main clinical aspects and treatment response of this condition during childhood.

Ofuji disease: a rare dermatosis and its challenging therapeutic approach

Abstract: Eosinophilic pustular folliculitis (EPF) or Ofuji disease is a rare dermatosis, prone to recurrence and chronicity. The peak incidence occurs in the third decade of life and its exact etiology remains unknown. Evidence suggests that the expression of adhesion molecules and the production of cytokines activate the follicular unit, but the stimulus that triggers these changes remains unclear. The three clinical variants reported in the literature include classic EPF, immunosuppression-associated EPF, and infancy-associated EPF. We report a case of eosinophilic pustular folliculitis with peculiar epidemiological characteristics, which represents a challenging therapeutic scenario.

Enfermedad de Sneddon-Wilkinson / Sneddon-Wilkinson disease

Introducción: la dermatosis pustulosa subcórnea o enfermedad de Sneddon-Wilkinson, es una enfermedad poco común, que se distingue por pústulas flácidas, recurrentes y coalescentes; su mecanismo etiológico no está del todo dilucidado, pero se relaciona con la presencia de enfermedades neoplásicas o inmunológicas. Presentación del caso: se presenta el caso de un niño de 4 años de edad, que acude a consulta de Dermatología del Hospital Andino de Chimborazo, con manifestaciones clínicas que permiten hacer el diagnóstico de dermatosis pustulosa subcórnea. Conclusiones: la enfermedad de Sneddon-Wilkinson es una afección rara que se presenta frecuentemente en pacientes femeninas de 40 años y más. Se caracteriza por una amplia diversidad de manifestaciones cutáneas que dificultan el pronóstico y la evolución del paciente. Esta enfermedad, cuando se presenta en niños, puede confundirse con otras afecciones que cursan con exantemas cutáneos(AU)

Introduction: subcorneal pustular dermatosis or Sneddon-Wilkinson disease is a rare illness characterized by recurrent, coalescent and flaccid pustules; its etiological mechanism is not totally known, but it is related to neoplastic or immunological diseases. Case presentation: a four-year old child who went to the dermatology service of Hospital Andino de Chimborazo. He presented with clinical manifestations that allow making the diagnosis of subcorneal pustular dermatosis. Conclusions: Sneddon-Wilkinson disease is a rare illness that frequently occurs in 40 years-old and older women. It has a wide range of skin manifestations that makes prognosis and recovery of patient difficult. When it appears in children, it may create confusion with other illnesses having skin exanthemas(AU)

Lúpus eritematoso sistêmico bolhoso em gestante: relato de caso / Bullous systemic lupus erythematosus in a pregnant woman: a case report

O lúpus eritematoso sistêmico pode apresentar inúmeras lesões cutâneas. As lesões bolhosas específicas do lúpus, apesar de raras, apresentam características clínicas e imunopatológicas próprias e implicam em diagnóstico diferencial entre inúmeras patologias bolhosas que podem sobrepor-se ao lúpus eritematoso sistêmico. Apresenta-se um caso de lúpus eritematoso sistêmico bolhoso em gestante.

Systemic lupus erythematosus (SLE) can cause numerous skin lesions. Despite being rare, lupus-specific bullous lesions demonstrate characteristic clinical and immunopathological features and require differential diagnosis among numerous bullous conditions that may overlap with SLE. The present study presents a case of bullous systemic lupus erythematosus (BSLE) in a pregnant woman.

Pustulosis subcórnea (enfermedad de Sneddon-Wilkinson): Caso clínico / Subcorneal pustular dermatosis: Report of one case

Background: We report a 35-year-old female patient with a one year history of a pustular and painful erythematous dermatitis, located in great folds, pubis and abdomen. She was evaluated in primary health care, receiving antifungal treatment, antimicrobials, topical and systemic non-steroidal anti-inflammatory drugs, with no response. A skin biopsy was compatible with subcorneal pustular dermatosis. She was initially treated with prednisone (0.8 mg/kg), observing a partial response. Therefore clotrimoxazole was initiated, obtaining an excellent response after 30 days of treatment.

Dermatose por IgA e IgG linear: relato de caso com boa resposta terapêutica à dapsona e ao micofenolato mofetil / Linear IgA/IgG bullous dermatosis: successful treatment with dapsone and mycophenolate mofetil

Relata-se o caso de paciente feminina, de 21 anos, com dermatose por IgA e IgG linear. Inicialmente, a resposta clínica foi favorável à dapsona. Após a interrupção desta medicação, por crise de anemia sintomática, precipitada por malária, houve piora da doença, apesar da utilização da prednisona e pulsoterapia com metilprednisolona. A reintrodução da dapsona, associada ao micofenolato mofetil, possibilitou o controle da enfermidade.

A 21-year-old female presenting linear IgA and IgG disease initially responded well to dapsone therapy. However, the treatment with dapsone was withdrawn due to severe anemia induced by malaria, which led to worsening of the clinical picture. Although prednisone and methylprednisolone were tried, the patient responded only to the association of dapsone and mycophenolate mofetil.

Lúpus eritematoso sistêmico bolhoso: diagnóstico diferencial com dermatite herpetiforme / Bullous systemic lupus erythematosus: differential diagnosis with dermatitis herpetiformis

O lúpus eritematoso sistêmico bolhoso é um subtipo raro do lúpus eritematoso sistêmico, que ocorre ainda de forma mais incomum nos pacientes pediátricos. Relatamos o caso de uma adolescente de 12 anos, apresentando lesões vésico-bolhosas em face, pescoço, tronco, mucosas oral e genital, anemia, leucocitúria estéril, FAN: 1/1280 padrão nuclear pontilhado grosso, Anti-Sm e Anti-RNP positivos. O estudo anatomopatológico sugere dermatite herpetiforme e a imunofluorescência direta revela IgG, IgA e fibrina ao longo da zona de membrana basal. Apresentamos um caso típico de lúpus eritematoso sistêmico bolhoso e enfatizamos a importância do diagnóstico diferencial com a dermatite herpetiforme.

Bullous systemic lupus erythematosus is a rare subset of systemic lupus erythematosus that is even rarer in pediatric patients. We report a case of a 12-year-old girl who presented with a vesiculobullous eruption on her face, neck, trunk and genital and oral mucosa, as well as anemia, sterile pyuria, ANA (1:1280, speckled pattern) and positive anti-Sm and anti-RNP. Pathological examination suggested dermatitis herpetiformis, and direct immunofluorescence revealed IgG, IgA and fibrin in the epithelial basement membrane zone. We present a typical case of bullous systemic lupus erythematosus and emphasize the importance of clinical and histopathological differential diagnosis with dermatitis herpetiformis.

Pustulosis subcórnea de Sneddo-Wilkinson / Subcorneal pustulosis of Sneddon-Wilkinson

Presentamos dos pacientes con pustulosis subcórnea de Sneddon Wilkinson diagnosticados en el Servicio de Dermatología del Hospital de Pediatría “Prof. Dr. Juan P. Garrahan”. Al examen físico se observaban múltiples pústulas fláccidas que asentaban sobre una piel eritematosa; en uno de los casos la distribución era generalizada a predominio de pliegues y parte proximal de los miembros, mientras que en el otro las lesiones se encontraban localizadas en la cula subcórnea con infiltrado a predominio neutrofílico en el interior, con inmunofluorescencia directa (IFD) negativa. El tratamiento inicial instaurado fue dapsona. Uno de ellos también requirió tratamiento con corticoides locales con múltiples recidivas de sus lesiones. Destacamos la escasa frecuencia de esta entidad en la niñez, las diferentes respuestas al tratamiento instaurado y la importancia del seguimiento de estos pacientes.

Efectividad del tratamiento tópico con esteroides en enfermedades vesículo-ampollares con signos de gingivitis descamativa: revisión de la literatura / The effectiveness of topical steroid treatment of vesiculo-bullous diseases with signs of desquamative gingivitis: a review of the literature

Existe una gran variedad de enfermedades que afectan las membranas mucosas las cuales incluyen la mucosa gingival. El signo de gingivitis descamativa constituye una condición que se caracteriza por la presencia de lesiones eritematosas, erosivas, atróficas y dolorosas en la encía marginal extendiéndose a otras localizaciones como la mucosa alveolar, palatina y yugal, entre otras. El manejo terapéutico de ésta alteración se basa principalmente en la administración de corticosteroides sistémicos y tópicos de alta potencia, al igual que el manejo dental apropiado. Es de importancia el control médico odontológico de éstos pacientes para garantizar la salud dental y sistémica del mismo. El propósito de ésta revisión es determinar, basándose en lo publicado en la literatura actual, hasta que punto es efectivo el tratamiento con corticosteroides tópicos lo que actualmente conocemos como signo de gingivitis descamativa

Prevalence of onychomycosis in patients with autoimmune diseases.

BACKGROUND: Onychomycosis is the most common nail disorder in adults. Many studies reported a higher prevalence of onychomycosis among particular patients, such as those with diabetes, poor peripheral circulation or immunosuppression. However, studies of the prevalence of onychomycosis in autoimmune patients who carry many of these predisposing factors have been limited OBJECTIVE: Study the prevalence of onychomycosis in autoimmune compared to non-autoimmune female patients. MATERIAL AND METHOD: A cross-sectional study of the prevalence of onychomycosis in autoimmune patients and non-autoimmune female patients visiting a dermatology clinic over a period of 18 months. One hundred and sixty-five female autoimmune patients were enrolled. RESULTS: The prevalence of onychomycosis in autoimmune patients was 10.2% (95%CI 6.5%, 15.9%) compared to 6.7% (95%CI 3.8%, 11.6%), in non-autoimmune patients (p > 0.05, 2-sided). Of vesiculobullous patients, mainly presenting with pemphigus and who were mostly on immunosuppressive medication, 24% had onychomycosis [p = 0.013; OR 4.39 (95%CI 1.27, 14.89)]. CONCLUSION: Exposure to humid microenvironments was an important factor in the occurrence of onychomycosis (p < 0.05, 2-sided). However, the number of patients with each individual disease was too small to conclude a prevalence of onychomycosis in conjunction with these individual cutaneous autoimmune diseases.

Ichthyosis bullosa of Siemens: response to topical tazarotene.

In 1937, Siemens described a Dutch family with superficial blistering, flexural hyperkeratosis, and characteristic mauserung appearance. Since then, less than 20 kindreds with this condition have been described in the English dermatologic literature. A 14-year-old boy presented with history of recurrent blistering and peeling of skin since the age of 1 month, predominantly seen over limbs and trunk, often associated with secondary infection. His mother also had similar symptoms from childhood. On examination, the child had typical mauserung peeling of the skin and dirty gray hyperkeratosis in a rippled pattern over flexures. Skin biopsy from the boy showed intracorneal blistering with epidermolytic hyperkeratosis in the upper spinous layers. The typical history and clinical features along with characteristic histological findings confirmed our diagnosis of ichthyosis bullosa of Siemens. It must be differentiated from other conditions with epidermolytic hyperkeratosis and skin peeling, such as bullous ichthyosiform erythroderma of Brocq and peeling skin syndrome. Our patient responded well to 0.05% topical tazarotene gel over four weeks.

Subcorneal pustular dermatosis in a Sudanese patient

Subcorneal pustular dermatosis [SPD] is a rare, benign, relapsing pustular eruption of unknown etiology. It usually develops in elderly women. Herein we describe a 23-yearold Sudanese male diagnosed with SPD, based on clinical and histological findings. The presentation is unusual; pustules involving the whole body sparing palms, soles, axillae and groin. The patient recovered completely after 8 weeks treatment of 100mg/day oral dapsone

Immunobullous diseases in Thai children: report of 24 cases.

BACKGROUND: Acquired immunobullous diseases in children are very rare and difficult to distinguish clinically. OBJECTIVE: To study the clinical manifestations, immunopathologic features, treatment and outcome of immunobullous diseases in Thai children. MATERIAL AND METHOD: The authors reviewed 24 cases of immunobullous diseases in children under 18 years at Queen Sirikit National Institute of Child Health from 1983 to 2000. Diagnosis of all cases was made by clinical presentations of chronic blistering diseases and confirmed by histopathology and immunofluorescent studies. RESULTS: There were 18 cases of chronic bullous diseases of childhood (CBDC), 4 cases of bullous pemphigoid (BP) and 2 cases of pemphigus vulgaris (PV). The mean age of onset of CBDC and BP were 4 years and 2 years respectively. There was an equal male to female ratio in both CBDC and BP. Both cases of pemphigus (neonate and 4 years old) were female. Most CBDC patients (18 cases) responded well to dapsone therapy although 2 cases had to be treated with prednisolone simultaneously. All cases with BP were treated successfully with prednisolone and dapsone. Neonatal pemphigus was treated symptomatically without steroid therapy. The second case of oral pemphigus was controlled with low dose prednisolone. CONCLUSION: Immunobullous diseases are very rare in children. All patients improved with corticosteroid and/or dapsone therapy.

Enfermedad por IgA lineal: a propósito de un caso / Lineal IgA disease: case report

Se presenta un caso de enfermedad por IgA lineal en un niño de 9 años de edad, con ampollas localizadas en zona axilar, glútea, genital y mucosa labial. El diagnóstico fue confirmado por histopatología, observándose ampolla subepidérmica, y por inmunofluorescencia indirecta, donde se detectó IgA lineal en la zona de la membrana basal. Las lesiones involucionan al tratamiento con sulfonas

A fatal case of erythema necroticans.

Erythema nodosum leprosum (ENL) classically presents as tender, erythematous nodules over the face, arms and legs. Severe ENL can become vesicular or bullous and break-down and is termed erythema necroticans (Jopling & McDougall, 1996) and is treated with corticosteroids. The causes of death in a majority of leprosy patients are the same as in the general population, with the exception of renal damage in lepromatous leprosy. There is possible increased mortality from side-effects of antileprosy drugs, steroids, or other drugs used in reactions, from toxaemia in severe reactions, and from asphyxia due to glottic oedema (Jopling & McDougall, 1996). We report here a case of erythema necroticans, the cause of death being septicaemia, secondary to skin ulcers and urinary tract infection, precipitated by corticosteroids.